Abstract
BackgroundAcute confusional state associated with migraine in adults is an infrequent entity. Around 30–60% of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients get affected by migraine attacks—the majority with aura—often as the first symptom of the disease. Acute confusional state during migraine has been rarely described in CADASIL patients and a complete neuropsychological assessment during the acute phase has never been conducted so far. Case summaryWe here describe the clinical and neuropsychological features of two distinct episodes of ACM in a 54-year-old female with CADASIL. EEG recording during acute confusional migraine and after attack resolution and neuroimaging has been reported. Discussion and literature reviewThis paper also reports a literature review on the topic of ACM in CADASIL highlighting a lack of adequate knowledge about this entity among clinicians and prompting further larger studies to explore its incidence and characteristics.
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